|CASE IN POINT - CLINICS IN URO-RADIOLOGY
|Year : 2018 | Volume
| Issue : 4 | Page : 262-264
Renal agenesis, blind ending ureteral remnant, and ectopic ureterocele
Aanchal Bhayana, Swarna Jain
Department of Radiodiagnosis, Vardhman Mahavir Medical College and Safdarjung Hospital, New Delhi, India
|Date of Web Publication||29-Oct-2018|
Department of Radiodiagnosis, Vardhman Mahavir Medical College and Safdarjung Hospital, New Delhi
Source of Support: None, Conflict of Interest: None
We wish to highlight the importance of multi-modality imaging in patients with renal agenesis who may present with lower urinary tract obstruction symptoms. Our case features a rare entity comprising three associated urinary tract anomalies, namely, a blind ending ureter, ectopic uretrocele, and ipsilateral renal agenesis, in a 20-year-old male patient.
Keywords: Blind ending ureter, ectopic ureterocele, renal agenesis,
|How to cite this article:|
Bhayana A, Jain S. Renal agenesis, blind ending ureteral remnant, and ectopic ureterocele. Astrocyte 2018;4:262-4
| Introduction|| |
Adult simple ureteroceles and unilateral renal agenesis are not that uncommon anomalies in isolation; however, concomitant presence of ureterocele, renal agenesis, and a blind ureteral remnant is a very rare occurrence. To the best of our knowledge, only five cases have been reported previously in literature with all these three concomitant features.,,,, Moreover, in our case, we observed ectopic ureterocele in adult, which is again a rare occurrence both in isolated setting and together with the presence of renal agenesis and blind ureteral bud.
| Case Report|| |
A 20-year-old male patient presented to the urology department, with history of occasional right-sided pelvic pain and intermittent urinary obstruction. Blood investigations and urine routine microscopy reports were normal. On clinical examination, there was mild right pelvic tenderness. The patient was referred to the radiology department for ultrasound.
Transabdominal ultrasound revealed absent right kidney in the right renal fossa and in the entire area of renal ascent [Figure 1]. Left kidney was normal. In addition, an elongated tubular structure was noted in the right lower quadrant of abdomen, which was distally ending into a cystic lesion within urinary bladder [Figure 2]a. This cystic lesion further appeared to communicate posteroinferiorly with prostatic urethra [Figure 2]b. For further evaluation of the entire urinary tract anomalies and better characterization of this tubular-cystic lesion, computed tomography (CT) urography was performed.
|Figure 1: Ultrasound Abdomen Images Show Absent Right Kidney With Empty Right Renal Fossa (a), and Normal Left Kidney (b).|
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|Figure 2: (a and b) Ultrasound Pelvis Images Show an Anechoic Tubular Structure (Red Arrow), Between Urinary Bladder and Rectum, with Cystic Protrusion into the Lumen of Bladder. (a), The Cystic Lesion Appears to Communicate with the Prostatic Urethra in the Midline. (b, Yellow Arrow).|
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CT study showed absent right kidney with a normally functioning left kidney [Figure 3]. Left ureter was normal [Figure 4]. Right ureter was not opacified. Instead, there was a tubular hypodense structure, on the right side of pelvis, with its blind proximal end. Distally, this structure was seen to expand into a cystic structure within the urinary bladder. Furthermore, inferiorly there appeared a suspicious communication of this cystic lesion with prostatic urethra [Figure 5]. The CT features suggested a possible diagnosis of right renal agenesis, a blind ending proximal end of right ureter with ectopic ureterocele.
|Figure 3: CT Urography Images in Axial. (a), and Coronal Planes. (b), Show Normal Left Kidney (Yellow Arrows), with Non Visualized Right Kidney.|
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|Figure 4: CT Urography Images in Both Axial and Coronal Planes (a and b), Show Normal Left Ureter. (a, Yellow Arrow), with Normal Orthotopic Insertion of Left Ureter at Left VUJ. (b, Red Arrow). Volume Rendered CT Images in Coronal and Coronal Oblique Planes. (c and d), Show Non Visualized Right Kidney and Right Ureter, with Normal Left Kidney and Left Ureter. (c), Left Ureter Shows Normal Orthotopic Insertion at Left VUJ (d; Black Arrow).|
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|Figure 5: Axial CT Urography Images Show Normal Left Ureter. (a, Red Dotted Arrow), with a Tubular Hypodense Structure Between Urinary Bladder and Rectum. (a, Yellow Arrow), with its Blind Cranial End. (b, Red Solid Arrow). Inferiorly, the Tubular Structure Shows Cystic Expansion Between the Urinary Bladder and Seminal Vesicles with Protrusion into Lumen of Bladder. (c, White Arrow). Further Inferiorly, this Cystic Lesion Appears to Open into the Prostatic Urethra in the Midline. (d, Yellow Dotted Arrow).|
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Therefore, for final confirmation of etiology, magnetic resonance imaging (MRI) was performed on a 1.5-T Phillips Achieva scanner. T1-weighted images were acquired in axial and coronal planes, whereas T2-weighted images were acquired in axial, coronal, and sagittal planes.
MRI images demonstrated right renal agenesis and a right pelvic tubular elongated structure, in line with the expected course of right ureter. This structure was hypointense on T1-weighted images and hyperintense on T2-weighted images. The cranial end of this tubular structure was blind. However, distally it showed a cystic protrusion with the lumen of urinary bladder and was further seen to communicate and insert into the prostatic urethra [Figure 6]. Left kidney, left ureter, and urinary bladder were normal.
|Figure 6: Coronal T2 Weighted MRI Image Shows Normal Left Kidney with Absent Right Kidney. (a), Axial T2 Weighted MRI Image Shows a Hyperintense Structure Between Urinary Bladder and Seminal Vesicles, with Cystic Protrusion into Bladder Lumen. (b, Yellow Arrow). Coronal T2 Weighted MRI Image Shows the Communication of this Hyperintense Structure with the Prostatic Urethra in the Midline. (c, Red Dotted Arrow).|
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Based on these imaging features, a radiological diagnosis of right renal agenesis with blind ending right ureteric bud and ectopic right ureterocele was made. The ureterocele bulging into the prostatic urethra was thought to be the cause of patient's symptoms.
| Discussion|| |
Renal agenesis results from failure of induction of metanephric blastema by ureteral bud, either due to maldevelopment of ureteral bud or faulty development of mesonephric duct. Renal agenesis is usually asymptomatic; however, renal agenesis is reportedly associated with other genitourinary abnormalities.
The blind ending ureteral remnants, seen along with ipsilateral renal agenesis, are dilated tubular structures seen on imaging, inserting either normally or at ectopic sites. These structures are thought to arise either from supernumerary ureteric buds or from lack of normal connection between the ureteric bud with metanephrogenic cap.
Ureterocele can be either simple or ectopic. Simple ureterocele shows normal ureteral insertion at the orthotropic site, whereas there is abnormal insertion of the ureter in ectopic variety. Ureterocele in adults is usually reported to be an isolated urinary tract abnormality and has a normal ureteral insertion at the vesicoureteral junction (VUJ).
Although adult simple ureteroceles and unilateral renal agenesis are not that uncommon anomalies in isolation, concomitant presence of ureterocele, renal agenesis, and a blind ureteral remnant is a very rare occurrence, especially in adults. This patient of ours had renal agenesis and presented with symptoms of urinary track obstruction due to ureterocele. To the best of our knowledge, only five cases have been reported previously in literature with all these three concomitant features.,,,, Moreover, in our case, we observed ectopic ureterocele in adult, which is again a rare occurrence both in isolated setting and together with the presence of renal agenesis and blind ureteral bud.
| Conclusions|| |
Detailed imaging evaluation can help in early and appropriate diagnosis in cases with associated anomalies in patients with renal agenesis. The possible differentials of cystic lesion in pelvis with associated renal agenesis include seminal vesicle cyst, wolffian para-vesicle cyst, Mullerian duct remnant, or ectopic ureterocele. Detailed multi-modality examination can help reveal the true nature of the lesion. Seminal vesicle cysts are seen arising from the seminal vesicles and usually appear hyperintense on T1/T2 WI due to proteinaceouscontents. Mullerian duct remnants arise from the midline of prostate. MRI can provide accurate anatomical detail and soft tissue resolution and help in problem-solving.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]